Vagal Nerve Stimulation for Refractory Epileptic Encephalopathy in Patient with Lissencephaly: Case Report from Indonesia

Abstract

Introduction: Lissencephaly is a rare brain malformation that causes severe developmental delays and intractable seizures. Traditional anti-epileptic drugs (AEDs) often fail to control seizures in these patients. Vagal nerve stimulation (VNS) has shown promise as an adjunctive treatment for refractory epilepsy.

Case Presentation: A 19-year-old male with lissencephaly and epileptic encephalopathy was referred for VNS implantation after experiencing poorly controlled seizures despite maximum doses of Levetiracetam and Valproic Acid. The patient had daily seizures and frequent episodes of convulsive status epilepticus. EEG revealed multifocal spikes, primarily in the frontal regions. VNS implantation was performed, initially set at 0.2 mA and gradually increased to 1.5 mA over two months. Follow-up results showed a progressive reduction in seizure frequency. By two months, the patient experienced 5-7 seizure-free days each month, with further improvements noted at six months. At ten months, the seizures were brief and infrequent, and the patient no longer required rescue medication.

Conclusion: VNS therapy significantly improved seizure control and the patient’s quality of life. Regular follow-up and tailored adjustments to the VNS settings were essential for optimizing seizure management. This case highlights VNS as a valuable option for managing refractory epilepsy in patients with severe neurological conditions.